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 277,896 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 58-year-old woman presented with a history of premature onset of menopause, longstanding hepatosplenomegaly, monoclonal gammopathy, lower limb polyneuropathy of recent onset, diabetes mellitus, excessive perspiration and leg edema. Polyneuropathy and excessive perspiration improved following a course of prednisone and melphalan. The clinical and pathophysiological features fit the rare entity known as POEMS syndrome.
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PMID:An unusual case of POEMS syndrome. 357 Jul 37

POEMS syndrome is a rare systemic multi-organ disease usually reported in Japanese patients. The clinical course is slow with a 5-year survival of 60%. Death is caused by polyneuropathy and or anasarka. We observed four cases in our ward (all males, aged 39, 57, 54 and 54 years) who all presented at least four characteristic clinical signs. Gynecomasty together with impotency was seen in all patients. All had hypogonadism and borderline hyperprolactinaemia. Hyperoestrogenism was seen in two. In three patients, rare endocrinopathies were part of the POEMS syndrome. One patient had diabetes mellitus. The clinical course was variable and a function of the effectiveness of plasmocyte dyscrasia therapy. Little work has been done on endocrinopathies in POEMS syndrome. In most cases, gonadotrope function is impaired with gynecomasty and impotency in men, amenorrhoea in women. Generally the hypogonadism is hypogonadotropic. Hyperoestrogenism is frequent and prolactin levels are normal or high with an exaggerated response to thyroid releasing hormone stimulation. The aetiology of POEMS syndrome is unknown. Current research is based on an immunologic theory based on the discovery of high levels of interleukin 6 in POEMS patients with or without Castleman's disease. The cytokine would affect the different organs and lead to clinical expression. Corticosteroids are usually effective in most patients, particularly in reducing the oedema and controlling the polyneuropathy.
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PMID:[Endocrine diseases in POEMS syndrome. Apropos of 4 cases]. 797 57

Crow-Fukase syndrome is a rare multiorgan disorder. Although renal disorders, such as proteinuria, and renal impairment, have been observed in half the cases of this syndrome, there have been few reports describing the renal lesions. We report here a case of this syndrome associated with membranoproliferative glomerulonephritis. A 43-year-old woman was referred to our hospital because of hyperglycemia. She had also been suffering from hyperpigmentation, hepatosplenomegaly, lymphadenopathy, polyneuropathy and endocrine dysfunction, including diabetes mellitus and amenorrhea. Serum electrophoresis showed M protein and immunoelectrophoresis revealed IgA (lambda). Bone marrow aspiration showed a slight increase in the number of plasma cells. Urine protein was 30 mg/dl, BUN was 17 mg/dl and creatinine 0.8 mg/dl. Light microscopic examinations showed enlargement of glomeruli with proliferation of mesangial cells and matrix, a lobular pattern of the glomeruli and thickening of the glomerular basement membrane and associated double contour. Electron microscopic examinations showed thickened capillary walls, associated mesangial interposition and subendothelial dense deposits. Moreover, fine granular deposits of IgM, C3, and fibrinogen along the basement membrane were observed on immunofluorescent studies.
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PMID:[A case of Crow-Fukase syndrome associated with membranoproliferative glomerulonephritis]. 807 25

POEMS syndrome has been defined as an association of plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. Although certain authors do not distinguish this syndrome from osteosclerosing myeloma, syndromes comparable to POEMS have been observed without bone lesions. Others have described the clinical features involved under the terms of Crow-Fukase's syndrome, PEP syndrome (pigmentation, oedema, plasma cell dyscrasia, or Takatsuki's syndrome. Seen in men twice as often as in women, usually between the ages of 40-50 years, all five clinical features are not always present or may be accompanied by other signs. The first sign of the peripheral polyneuropathy is usually sensorial impairment followed by distal then proximal motor deficit. The deficit is usually severe and 50% of the patients become unable to walk. Cranial nerves are rarely involved. Liver, spleen and lymph node enlargement are observed. The most frequent signs of an endocrinopathy are gynaecomastia, atrophy of the testicules, impotence and amenorrhoea. Testosterone levels are low and oestrogen levels are increased in men together with luteinizing hormone, follicle stimulating hormone and prolactin. Hypothyroidism is frequent and diabetes mellitus is observed in 50% of the patients. Protein M is a monoclonal immunoglobulin (IgG or IgA), almost always with a light lambda chain. Skin changes include hyperpigmentation, hypertrichosis, hyperhidrosis, thickening of the skin suggestive of sclerodermia and papillary angiomas. Other signs, especially peripheral oedema often occur early in the disease course and may precede the peripheral neuropathy. POEMS syndrome is often associated with a myeloma (up to 50% of the cases in certain series). Although immunoglobulin deposit on myelin sheaths, anti-endocrine antibodies and receptors of lambda chains have been proposed as playing a role, no mechanism of pathogenesis has been determined. The natural history of the disease leads to a severe polyneuropathy. The patients become totally bedridden and death results from complications of decubitus rather from the direct effect of the underlying dyscrasia. When bone lesions are minor, radiotherapy or surgery can improve the neuropathy and resection of a solitary plasmocytoma can lead to total remission. Chemotherapy or corticosteroids may improve the polyneuropathy in certain cases. Plasma exchange has not been successful.
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PMID:[POEMS syndrome]. 807 60

Castleman disease, or angiofollicular lymph node hyperplasia, and POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes), are associated and can lead to a clinical conundrum. The physician caring for a patient with Castleman disease should be alert to the development of multiple endocrine deficiencies, including primary hypogonadism, diabetes mellitus, hypothyroidism, and adrenal insufficiency. Avoidance of treating hypothyroidism alone when there is concomitant subclinical adrenal insufficiency is important, to avoid precipitating an adrenal crisis. A better outcome may result from earlier recognition of the endocrinopathies of this syndrome. This article describes a patient with Castleman disease in whom the features of POEMS unfolded over the ensuing years.
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PMID:Case report: Castleman disease in association with POEMS. 814 Nov 36

The POEMS syndrome is a synopsis of different symptoms such as polyneuropathy, organomegaly, endocrine disturbances, M-protein and skin changes. The leading symptoms are neuropathy and the skin symptoms. Additionally, a monoclonal light chain gammopathy is often found. The administration of immunosuppressive drugs yields a substantial improvement in some cases. We report here about a 72 year old lady who fell ill with a rapidly progressive neuropathy accompanied by hyperpigmentation and a morphea-like induration of the skin. A biopsy of the sural nerve showed a demyelinating axonal neuropathy and a focal vasculitis. Isoelectric focussing revealed oligoclonal bands in cerebrospinal fluid and serum. The cortisol serum level was very low and there were signs of a latent diabetes mellitus. These clinical features correspond to the POEMS syndrome. The prescription of initially 1 mg and later 0.5 mg prednisone improved the patient's condition dramatically.
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PMID:[POEMS syndrome: a contribution to differential diagnosis of polyneuropathy]. 850 14

POEMS syndrome is an uncommon association of polyneuropathy, organomegaly (liver, spleen, lymph nodes), endocrinopathy (hypogonadism primary hypothyroidism, diabetes mellitus, adrenal insufficiency), monoclonal gammopathy and skin changes; bone lesions (generally osteosclerotic, less frequently osteolytic or mixed) are nearly always present in this multisystem disease. We describe the first case of a female Italian patient with POEMS syndrome. Therapy with prednisone was of scant benefit and the patient died of cardiogenic shock some days after temporary pacemaker implantation, performed for recurrent episodes of bradycardia. From the beginning of steroid therapy she survived about fifteen months.
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PMID:POEMS syndrome in a female Italian patient. A case report. 853 76

POEMS syndrome is a systemic disorder with peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. The association of POEMS syndrome with lympho-proliferative disorder is very commun. The pathogenesis remains poorly understood but implication of cytokines (interleukins 1 and 6) is suspected. We report a case of a classic POEMS syndrome (with polyneuropathy, hepatomegaly, diabetes melitus, hyperpigmentation, monoclonal IgG lambda, anasarca and solitary plasmocytoma), associated with high serum levels of interleukin 6.
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PMID:[POEMS syndrome: role and value of interleukin-6]. 878 86

The clinical relevance of neurological disorders associated with impaired glucose tolerance(IGT) is reviewed. In this review some neurological diseases, such as, myotonic dystrophy, Crow-Fukase syndrome, Wolfram syndrome (DIDMOAD), Friedreich ataxia, spinal muscular atrophy of the Kennedy-Alter-Sung type, amyotrophic lateral sclerosis, Parkinson-dementia, and MELAS are discussed in relation to, glucose intolerance. Although the etiology of these disorders still remains an enigma, MELAS was caused by an A-to-G mutation at nucleotide position 3243 of the mitochondria genome. An association of "diabetic neuropathy" with IGT appears to be negative. Peripheral nerve function did not differ between IGT and control subjects, whereas autonomic nerve function deviated; an abnormal expiration to inspiration ratio of R-R interval was significantly more common in IGT than in control subjects. In conclusion, diabetes, but not IGT, is associated with peripheral nerve dysfunction.
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PMID:[Neurological disorders associated with impaired glucose tolerance]. 891 31

POEMS syndrome is a rare synopsis of different multisystemic disorders (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammapathy, and skin lesions) associated with plasma cell dyscrasia. We herein report the atypical case of a 44-year-old white man presenting with glomerulopathy, POEMS syndrome, and erythema elevatum diutinum with a few-year history of non-insulin-dependent diabetes mellitus (NIDDM) and seronegative rheumatoid arthritis (RA) as early manifestations of IgAlambda multiple myeloma. The prescription of 1 mg/kg/day prednisone improved the patient's features dramatically. Skin lesions improved by the association of glucocorticoids and plasma exchange, recurred when plasmapheresis ceased, and remitted when plasma exchange was reintroduced. NIDDM requiring insulinotherapy recurred when corticoids were discontinued and remitted when prednisone was reintroduced. However, prednisone and plasmapheresis had no effect on polyneuropathy, M-paraprotein, and plasma cell dyscrasia in our patient, who developed indolent multiple myeloma a few years later. We thus concluded that POEMS syndrome, steroid-dependent diabetes mellitus, rheumatoid arthritis, RA, and skin vasculitis in our patient were triggered by plasma cell dyscrasia.
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PMID:POEMS syndrome, steroid-dependent diabetes mellitus, erythema elevatum diutinum, and rheumatoid arthritis as extramedullary manifestations of plasma cell dyscrasia. 1007 79


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